Scleroderma

WHY THE NAME?
The word scleroderma comes from two Greek words 'sclero' meaning hard and 'derma' meaning skin. Over two centuries ago in 1753, Curzio of Naples first described the condition but the current term scleroderma was named by Gintrac in 1847, when it was recognised as a skin disorder. In the early 20th century, reports suggested that the disease was a systemic disorder which could affect the internal organs of the body and not just the skin. In 1945 Goetz used the term progressive systemic sclerosis but as scleroderma is not always progressive, the term systemic sclerosis (SSc) is more widely used today.

SCLERODERMA
Scleroderma is an uncommon disease of the immune system, blood vessels and connective tissue. In this condition the skin, usually of the hands and feet, becomes stiff, tight and shiny. As a result of changes in these three areas, the body produces too much collagen. Collagen which is the major protein of the connective tissue, binds the body together and is found in the skin, blood vessels, joints and internal organs, such as the kidneys, heart, lungs and bowel. There are several types of collagen and different forms are found in different parts of the body. They are produced by cells called fibroblasts. When fibroblasts produce too much collagen it results in fibrosis, i.e. thickening.

The skin, usually of the hands and feet may become thickened, tough, tight and leathery. In addition to affecting the fingers, the fibrosis may spread to other areas and organs of the body. The arms, face, trunk and legs may be involved and movement of the limbs may become limited.

Damage to the blood vessels is usually seen in the form of severe Raynaud's, painful pits or scars on the finger tips, telangiectasia and ulcers. These are external signs of scleroderma but internal vascular damage may also occur.

The immune system in a human is composed of specialised cells (lymphocytes, monocytes). Their role is to ward off foreign substances by recognising that they are foreign and mounting an attack, whether it be a bacteria or virus, a chemical agent or a transplanted organ. When the immune system responds to and destroys substances that are not foreign but in fact healthy tissues, the result is an immune injury. In scleroderma this injury probably starts a process which becomes persistent and can be severe.

There are different types of the condition; diffuse cutaneous systemic sclerosis (dcSSc), limited cutaneous systemic sclerosis (lcSSc), scleroderma sine scleroderma and localised scleroderma (of which there are two types: morphoea and linear).

Diffuse Cutaneous Systemic Sclerosis (dcSSc)
Shortly after the onset of Raynaud's, the patient presents with skin changes (puffy or hidebound), has truncal skin involvement and 'creaking' tendons at joints e.g. wrists and elbows. Early lung disease, kidney, gastro-intestinal and heart involvement become evident, in some patients. There are changes in the nailfold capillary and certain antibodies may be present in the blood. A specific marker of scleroderma, usually the progressive diffuse form, is Scl-70 which is present in 30% of patients. Anyone with diffuse SSc needs to be checked frequently (at least every 6-12 months), in the first 5 years.

Limited Cutaneous Systemic Sclerosis (lcSSc)
Patients with limited SSc will have had Raynaud's for years, occasionally decades, and there will be skin involvement to the hands, face, feet and forearms. These patients used to be classified as having CREST. In the early 1900's several physicians noted a group of features often seen when they examined patients with scleroderma (systemic sclerosis). they coined the term CREST syndrome. This is now known as limited cutaneous systemic sclerosis. Patients with limited disease also need checking every 6-12 months, depending on the stability of the disease.

Scleroderma sine scleroderma
Scleroderma sine scleroderma is extremely rare. This form of scleroderma is unique in that patients can have any of the characteristic features of internal organ involvement WITHOUT having detectable skin features.Patients can have any of the reported features of disease of the bowels, oesophagus, lungs, heart, and/or kidneys. In an individual patient, treatment is customised according to the extent and severity of involvement of these internal organs.

Localised scleroderma
To download a leaflet on localised scleroderma click here.

WHO GETS IT?
Anyone of any age can develop scleroderma. It is four times more common in females than males and during childbearing years, the percentage rises to ten females to one male. Scleroderma usually begins between the ages of twenty-five and fifty-five and is usually preceded by Raynaud's. It occasionally occurs in children and the elderly, but fortunately most types of childhood scleroderma are localised and only involve the skin.

Although over 95% of patients with scleroderma have Raynaud's, the chances of someone with Raynaud's developing scleroderma is very small - it is less than 2% in females and 6% in males. The condition does not appear to be related to race, ethnic background or geographical area.

IS IT HEREDITARY?
Scleroderma cannot be directly inherited but there is some evidence that patients have genetic markers which predispose them individually towards the disease.

OCCUPATIONAL CAUSES
A scleroderma-like illness can be caused by various chemicals and drugs. The best known of these are vinyl chloride and silica and may affect people who work in the chemical industry.

For further information on specific aspects of scleroderma download our leaflets from here:

Scleroderma (Systemic Sclerosis)
Cutaneous Systemic Sclerosis (CREST)
Localised Scleroderma (Morphoea and Linear)
Lungs in Scleroderma
Gut in Scleroderma
Kidney Involvement in Scleroderma
Scleroderma in Children
Sexuality in Scleroderma
Foot Care in Raynaud's and Scleroderma
Skin Care & Digital Ulcers

Scleroderma - The Inside Story by Anne H Mawdsley MBE
This book is for people with scleroderma, their relatives and health professionals who wish to know more about the condition. It covers Raynaud’s phenomenon and most aspects of scleroderma. It is intended to guide patients through many of the symptoms associated with these conditions. It is hoped that this book will give some inspiration to people who may have felt isolated but want to know more about their condition and ways in which to cope with problems as and when they occur.
PRICE £5.75 (including p & p) available by sending a cheque made payable to ‘Raynaud’s & Scleroderma Association’ to: RSA, 112 Crewe Road, Alsager, Cheshire ST7 2JA